Journal
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
Volume 17, Issue 7-8, Pages 561-570Publisher
TAYLOR & FRANCIS LTD
DOI: 10.3109/21678421.2016.1173702
Keywords
Amyotrophic lateral sclerosis; magnetic resonance imaging; pathophysiology; diffusion tensor imaging; basal ganglia; biomarker
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Funding
- Irish Institute of Clinical Neuroscience (IICN) - Novartis Ireland Research Grant
- Iris O'Brien Foundation
- Perrigo Clinician-Scientist Research Fellowship
- Health Research Board
- Research Motor Neuron (RMN-Ireland) foundation
- European Community [259867]
- EU
- Perrigo Company plc
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A large multiparametric MRI study has been undertaken to evaluate anatomical patterns of basal ganglia, white matter and cortical grey matter involvement in ALS. Unaffected brain regions are mapped in patients with significant disability. Multiple white matter diffusivity measures, cortical grey matter density alterations, basal ganglia volumes and subcortical grey matter atrophy patterns are evaluated. Results demonstrated a strikingly selective anatomical vulnerability pattern in ALS that preferentially affects specific grey matter structures, commissural white matter tracts and basal ganglia regions, suggestive of networkwise neurodegeneration in ALS. In conclusion, ALS pathology exhibits predilection for selective and inter-connected anatomical sites that can be comprehensively characterized in vivo by multiparametric neuroimaging. The systematic characterization of unaffected brain regions in ALS has implications for the development of classifier analyses and elucidation of disease biology. The involvement and sparing of contiguous brain regions raises important pathophysiological, phylogenetic and ontogenetic questions regarding ALS pathogenesis and disease spread.
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